Pallidopyramidal Syndrome (PARK15)


Presentation

musculoskeletal
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  • gastrointestinal
    Muscle Rigidity
    • Causes include persistenttremor, muscle rigidity, dystonia, musculoskeletal injury (i.e., sprains, bruises, bone fractures resulting from a fall etc.), burns and inflammation.[de.slideshare.net]
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  • neurologic
    Tremor
    • Symptoms of Pallidopyramidal syndrome Pyramidal signs Parkinson disease Tremor Rigidity Hyperreflexia more symptoms...»[rightdiagnosis.com]
    • Involuntary movements are often non-suppressible (e.g., most tremors and myoclonus), but some can be partially suppressible (e.g., some tremors, chorea, dystonia, stereotypies and some tics) ( Koller and Biary, 1989 ).[neupsykey.com]
    • In the mother, resting tremor was associated with bradykinesia and parkinsonian gait, while in the brother the tremor was isolated and of the postural type.[docslide.com.br]
    • A 40-year-old housewife presented with tremors on the left side for two-and-a-half years. Five months later the tremor appeared in the right upper limb also.[neurologyindia.com]
    • FRAGILE X TREMOR/ATAXIA SYNDROME; FXTAS Is also known as ; Related symptoms: Inertia Diffuse cerebellar atrophy Obsessive-compulsive trait Impaired distal vibration sensation Saccadic smooth pursuit SOURCES: ORPHANET OMIM More info about FRAGILE X TREMOR[mendelian.co]
    Resting Tremor
    • In the mother, resting tremor was associated with bradykinesia and parkinsonian gait, while in the brother the tremor was isolated and of the postural type.[docslide.com.br]
    • IDIOPATHIC PD  UK Brain Bank Criteria  bradykinesia and at least one of the following: rest tremor, rigidity, or postural instability. 11.[slideshare.net]
    • Nonmotor symptoms — Nonmotor symptoms of Parkinson disease are those that are not related to movement. bradykinesia: slowness of movement and difficulty to initiate movement rigidity: stiffness of movement. resting tremor: involuntary shaking movement[de.slideshare.net]
    • Certain localizations within the basal ganglia are classically associated with specific movement disorders: substantia nigra with bradykinesia and rest tremor; subthalamic nucleus with ballism; caudate nucleus with chorea; and putamen with dystonia.[neupsykey.com]
    • […] developmental delay 40% Hyporeflexia 40% Cerebellar atrophy 40% Autosomal dominant inheritance 40% Dysphagia 30% Babinski sign 30% Dystonia 30% Anxiety 30% Intellectual disability 30% Impaired distal vibration sensation 30% Mitochondrial inheritance 30% Resting[mendelian.co]
    Hyperreflexia
    • Symptoms of Pallidopyramidal syndrome Pyramidal signs Parkinson disease Tremor Rigidity Hyperreflexia more symptoms...»[rightdiagnosis.com]
    • The pyramidal features include paraparesis, scissoring of gait, Bakinski sign, hyperreflexia, and pseudobulbar effect.[neurologyindia.com]
    • Supranuclear gaze palsy; Oculogyric dystonic spasms NEUROLOGIC: [Central nervous system]; Parkinsonism; Hypokinesia; Akinesia; Mask-like facies; Rigidity; Anarthria; Festinate gait; Facial-faucial-finger mini-myoclonus (FFF); Spasticity; Mild paraparesis; Hyperreflexia[genome.jp]
    • 70% Rigidity 70% Tremor 60% Autosomal recessive inheritance 60% Dysarthria 60% Try adding any of this symptoms in our app Other less frequent symptoms Patients with Muscle weakness and Bradykinesia. may also develop some of the following symptoms: 50% Hyperreflexia[mendelian.co]
    • […] compound heterozygous mutations) 25 and an excellent response to levodopa therapy.26,27 Over 200 mutations have been described (up to May 2015). 28 The phenotype is characterised by early dyskinesia in feet and legs, 29 motor fluctuations, symmetric onset, hyperreflexia[acnr.co.uk]
    Pyramidal Tract Signs
    • METHODS:: We performed clinical and genetic studies in two families with early-onset, progressive parkinsonism and pyramidal tract signs.[moh-it.pure.elsevier.com]
    • Horowitz & Greenberg (1975) were the first to report the dramatic reversibility, after L-dopa therapy, of the picture of paralysis agitans in PPS whereas the pyramidal tract signs did not (2).[docslide.net]
    • At the time of most recent exam- ination at age 16, the patient exhibited gait impairment, bradykinesia, and pyramidal tract signs.[dokumen.tips]
    Postural Instability
    • The patient presented repeated postural instability episodes in the upright position, with loss balance tendency and recurrent falls.[unboundmedicine.com]
    • IDIOPATHIC PD  UK Brain Bank Criteria  bradykinesia and at least one of the following: rest tremor, rigidity, or postural instability. 11.[slideshare.net]
    • Postural instability: leads to poor bland and tendency to fall Lead pipe rigidity: the resistance is present to the same degree throughout the full range of movement, affecting flexor and extensor muscle groups equally.[de.slideshare.net]
    Bilateral Babinski's Reflex
    Lethargy
    • Onset started with general weakness, muscle stiffness and lethargy and progressed rapidly to a deterioration of motor function accompanied by involuntary jerky movements, and total dependence for daily activities.[cags.org.ae]
    Babinski Sign
    • […] of the following symptoms: 50% Hyperreflexia 50% Gait ataxia 50% Parkinsonism 40% Ataxia 40% Ptosis 40% Nystagmus 40% Seizures 40% Global developmental delay 40% Hyporeflexia 40% Cerebellar atrophy 40% Autosomal dominant inheritance 40% Dysphagia 30% Babinski[mendelian.co]
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  • Face, Head & Neck
    Hypomimia
    • Pill-rolling tremor, hypomimia, hypophonia, stooping posture, striatal toes and equinovarous deformity may be noted. The pyramidal features include paraparesis, scissoring of gait, Bakinski sign, hyperreflexia, and pseudobulbar effect.[neurologyindia.com]
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  • Workup

    Treatment

    Prognosis

    Complications

    Wilson Disease
    • 2 5 Evaluation of Kayser–Fleischer ring in Wilson disease by anterior segment optical coherence...[jpgmonline.com]
    • Such a patient may simulate young onset parkinsonian disease, dopa-responsive dystonia and Wilson's disease.[neurologyindia.com]
    • Journal of Neuropathology & Experimental Neurology , Volume 13, Issue 1, 1 January 1954, Pages 168–180, View article Tags: connective tissue diseases , peripheral neuropathy The Histopathology of Wilson's Disease: A Study with Silver Carbonate K.[academic.oup.com]
    Secondary Parkinsonism
    • There are many causes of parkinsonism; they can be divided into four major categories: primary, secondary, parkinsonism-plus syndromes, and heredodegenerative disorders ( Table 1.8 ).[neupsykey.com]
    • SECONDARY PARKINSONISM  Infectious Post- encephalitic  HIV; SSPE; Prion diseases  Drugs  Dopamine Receptor Blocking drugs, Reserpine, Tetrabenazine, Alpha Methyl Dopa, Lithium, Flunarizine, Cinnarizine.  Toxins  MPTP, Carbon monoxide, Manganese,[slideshare.net]

    Etiology

    Causes

    Wilson Disease
    • 2 5 Evaluation of Kayser–Fleischer ring in Wilson disease by anterior segment optical coherence...[jpgmonline.com]
    • Such a patient may simulate young onset parkinsonian disease, dopa-responsive dystonia and Wilson's disease.[neurologyindia.com]
    • Journal of Neuropathology & Experimental Neurology , Volume 13, Issue 1, 1 January 1954, Pages 168–180, View article Tags: connective tissue diseases , peripheral neuropathy The Histopathology of Wilson's Disease: A Study with Silver Carbonate K.[academic.oup.com]

    Epidemiology

    Sex distribution
    Age distribution

    Pathophysiology

    Prevention

    Summary

    Patient Information

    Other symptoms


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    References

    1. FBXO7 gene mutations may be rare in Chinese early-onset Parkinsonism patients - L Luo, Q Xu, J Guo, L Wang, C Shi, J Wei, Z Long - Neuroscience , 2010 - Elsevier
    2. A pure parkinsonian syndrome following acute carbon monoxide intoxication - HL Klawans, RW Stein, CM Tanner - Archives of , 1982 - Am Med Assoc
    3. A pure parkinsonian syndrome following acute carbon monoxide intoxication - HL Klawans, RW Stein, CM Tanner - Archives of , 1982 - Am Med Assoc
    4. ATP13A2 mutations (PARK9) cause neurodegeneration with brain iron accumulation - SA Schneider, C Paisan‐Ruiz, NP Quinn - Movement , 2010 - Wiley Online Library
    5. FBXO7 gene mutations may be rare in Chinese early-onset Parkinsonism patients - L Luo, Q Xu, J Guo, L Wang, C Shi, J Wei, Z Long - Neuroscience , 2010 - Elsevier
    6. Anticholinergic drugs: response of parkinsonism not responsive to levodopa - H Yamada, T Momose, M Okada - Journal of Neurology, , 2002 - jnnp.bmj.com
    7. ATP13A2 missense mutations in juvenile parkinsonism and young onset Parkinson disease - A Di Fonzo, HF Chien, M Socal, S Giraudo - Neurology, 2007 - AAN Enterprises
    8. Advances in the Genetics of Human Tremor - F Coppedè - Mechanisms and Emerging Therapies in Tremor , 2013 - Springer
    9. Anticholinergic drugs: response of parkinsonism not responsive to levodopa - H Yamada, T Momose, M Okada - Journal of Neurology, , 2002 - jnnp.bmj.com
    10. A Clinical and Pathological Résumé of Combined Disease of the Pyramidal and Extrapyramidal Systems, with Especial Reference to a New Syndrome - J Lhermitte, D McAlpine - Brain, 1926 - Oxford Univ Press

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